ATRETIC PARIETAL CEPHALOCELES REVISITED - AN ENLARGING CLINICAL AND IMAGING SPECTRUM

PURPOSE: We describe imaging features that are clues to the diagnosis of atretic cephaloceles and discuss clinical findings and a possible m echanism by which these lesions develop. METHODS: Eight children (five girls and three boys) ranging in age from 1 day to 3 Sears 1 months w ith midline subscalp lesions underwent radiologic examination with CT or MR imaging. In all cases, the lesions were surgically excised and s ubjected to pathologic examination, Imaging studies and medical record s were reviewed retrospectively. RESULTS: Six of eight children had ve rtical embryonic positioning of the straight sinus with a prominent su perior cerebellar cistern. A ''spinning-top'' configuration of the ten torial incisura, a ''cigar-shaped'' CSF tract within the interhemisphe ric fissure, fenestration of the superior sagittal sinus, and ''peakin g'' of the tentorium were associated findings helpful in making this d iagnosis. Two of the eight children had findings indistinguishable fro m focal dermoid, six were developmentally normal, one had mild motor d elay, and one died at the age of 3 years. Pathologic examination revea led glial, meningeal (arachnoid), fibrous, and dermal elements. CONCLU SION: Characteristic findings on MR images and CT scans provide clues to the diagnosis of atretic cephalocele. However, even in the presence of abnormal imaging findings, these children may be developmentally n ormal.